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Target intelligence / Profile preview
Iron-sulfur cluster assembly enzyme ISCU, mitochondrial (ISCU)
- Target
- ISCU
- Molecular classification
- Enzyme, Scaffold protein, Mitochondrial protein, Iron-sulfur cluster transferase, NifU family member
01
Overview
Iron-sulfur cluster assembly enzyme ISCU, mitochondrial is a scaffold protein encoded by the ISCU gene. It is essential for the assembly and maturation of [2Fe-2S] and [4Fe-4S] iron-sulfur clusters, which serve as cofactors for numerous metabolic enzymes. ISCU functions in mitochondria, acting as a platform for cluster formation and subsequent delivery to target apoproteins, in cooperation with molecular chaperones such as HscA and HscB. Mutations leading to ISCU deficiency result in impaired mitochondrial energy metabolism and manifest clinically as mitochondrial myopathy, characterized by muscle weakness, myalgia, and exercise intolerance.
- Other names
- ISCUIscU iron-sulfur cluster scaffold homologISU2NIFUNIFUNHMLhnifUiron-sulfur cluster assembly enzymeiron-sulfur cluster scaffold homolog (E. coli)NifU-like N-terminal domain containing
02
Biological functions
Iron-sulfur cluster synthesis and maturationScaffold for iron-sulfur cluster assembly and transferSupport for metabolic enzymes (such as succinate dehydrogenase and aconitase)Regulation of metabolism, iron homeostasis, oxidative stress responseCritical for energy production in mitochondria
03
Disease associations
Mitochondrial myopathy with ISCU deficiency (exercise intolerance, tachycardia, muscle weakness, myalgia)Progressive muscle weakness and wasting due to enzymatic deficiencyOther metabolic deficits related to iron-sulfur cluster biosynthesis failure
04
Safety considerations
Deficiency leads to mitochondrial dysfunction, energy production deficits, and iron overload in muscle cellsMutations are associated with progressive muscle wasting and exercise intolerance
05
Biomarkers
Genetic mutations in ISCU (IVS5+382G>C, Gly50Glu) for diagnosis of ISCU deficiency/myopathyDecreased iron-sulfur cluster-containing enzyme activity (e.g., decreased succinate dehydrogenase, aconitase) in muscle biopsy
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